Recurrent Tolosa-Hunt Syndrome in the Setting of Uncontrolled Diabetes Type 1

Presentation Number: SAT 635
Date of Presentation: April 1st, 2017

Kimberly Kochersperger Lessard*, Catherine Anastasopoulou and George Newman
Einstein Medical Center, Philadelphia, PA

Abstract

Background: Tolosa-Hunt syndrome describes a rare, painful opthalmoplegia caused by an idiopathic granulomatous inflammation of the cavernous sinus or superior orbital fissure. Classic features of the syndrome include episodic retroorbital pain and ophthalmoplegia affecting the third, fourth, and/or sixth cranial nerves. It has been linked to autoimmune disorders and patients with longstanding poorly controlled diabetes.

Case: A 39-year-old female with a history of uncontrolled diabetes mellitus type 1 complicated by neuropathy and retinopathy presented with a sudden onset of left temporal headache accompanied by blurred vision of the left eye. Despite initial migraine treatment, the patient developed diplopia and progressive vision loss in her left eye. Ophthalmologic evaluation showed a pupil-sparing third nerve palsy. MRI revealed inflammatory changes infiltrating bilateral intraorbital fat planes. Additional workup, with the exception of HgbA1c 11.3%, was unremarkable and she was discharged with presumed third nerve palsy. A year later, she presented again with ptosis, painful opthalmoplegia of the right eye and binocular diplopia found to be from III and IV nerve palsies. MRI was significant for hyperenhancement and inflammatory changes of right superior medical recti and retrobulbar fat bilaterally. The recurrence and alternating laterality of her symptoms raised the suspicion for Tolosa-Hunt syndrome. A course of high dose prednisone was given with improvement in her pain and ptosis, but mild opthalmoplegia remained at 72 hours. One week following discharge, she experienced a relapse of right-sided headache and ptosis. This prompted treatment with high dose intravenous corticosteroids with subsequent slow tapering over weeks. Diabetic control was improved (HgbA1c 9.0%) and the patient has not had further recurrence.

Discussion: With an incidence estimated at one case per million per year, Tolosa-Hunt syndrome can affect both men and women of any age. Its symptoms can be mistaken for other conditions causing mass effect on the cavernous sinus such as tumors, aneurysm, thrombosis or dissection, as well as non-compressive conditions including migraine and giant cell arteritis. Its differentiation from other disorders is based on the clinical presentation in conjunction with neuroimaging results. Rapid resolution of pain and gradual regression of palsy with corticosteroid therapy helps confirm the diagnosis. Other testing, including CSF evaluation and surgical biopsy, are recommended if symptoms are unresponsive to steroids. Some patients will require other immunosuppressive medications either to limit the complications of corticosteroid use or to keep the disorder in remission. In many cases, as seen in our patient, optimized therapy of an underlying autoimmune disorder such as diabetes, can help improve the course of the disease and prevent recurrence.

 

Nothing to Disclose: KKL, CA, GN